Zusammenfassung
Die pulmonale Hypertonie (PH) ist eine schwerwiegende Erkrankung, die unbehandelt mit einer schlechten Prognose behaftet ist. Nach der aktuellen Klassifikation (Venedig, 2003) wird die pulmonal arterielle Hypertonie (PAH) von anderen Formen der PH abgegrenzt. Aktuelle Fortschritte in der medikamentösen Therapie haben in jüngster Zeit zu einer deutlichen Verbesserung der Versorgung von Patienten insbesondere mit PAH geführt. Daher sind die rechtzeitige Diagnosestellung sowie die exakte Klassifikation von zunehmender Bedeutung. Der vorliegende Übersichtsbeitrag bietet einen Überblick über die Definition, Klassifikation und Pathogenese sowie das klinische Erscheinungsbild verschiedener Formen der PH. Darüber hinaus werden die aktuellen Empfehlungen zur diagnostischen Abklärung sowie die derzeitigen Behandlungsoptionen insbesondere der PAH unter besonderer Berücksichtigung von Prostanoiden, Endothelinrezeptorantagonisten (ERA) und Phosphodiesterase-5- (PDE5-)Inhibitoren wie Sildenafil dargestellt. Schließlich werden neue Entwicklungen und Therapieansätze diskutiert, die derzeit ein dynamisches Feld der Grundlagenwissenschaft und klinischen Forschung darstellen.
Abstract
Pulmonary hypertension (PH) is a devastating disease that, if untreated, is characterized by a poor prognosis. According to the current classification (Venice, 2003), pulmonary arterial hypertension (PAH) is distinguished from other forms of PH. Recent advances in drug therapy have led to a dramatic improvement in medical care, particularly in patients with PAH. Hence, early establishment of the diagnosis and a precise classification appear increasingly important. This review provides an overview on the definition, classification, pathophysiology, and clinical presentation of various forms of PH. Furthermore, it summarizes the recommended diagnostic work-up and current treatment options, particularly for PAH, with special emphasis on prostanoids, endothelin receptor antagonists (ERAs), and phosphopdiesterase type 5 (PDE5) inhibitors such as sildenafil. Finally, novel developments are discussed which currently represent an exciting field of basic and clinical research.
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Dieser Beitrag wurde in modifizierter Form bereits in Clin Res Cardiol Suppl 2/2007 veröffentlicht. Mit freundlicher Genehmigung der Deutschen Gesellschaft für Kardiologie – Herz- und Kreislaufforschung e.V.
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Dumitrescu, D., ten Freyhaus, H., Wasserman, K. et al. Pulmonale Hypertonie. Pneumologe 4, 283–302 (2007). https://doi.org/10.1007/s10405-007-0159-7
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DOI: https://doi.org/10.1007/s10405-007-0159-7
Schlüsselwörter
- Pulmonale Hypertonie (PH)
- Pulmonal arterielle Hypertonie (PAH)
- Endothelinrezeptorantagonisten (ERA)
- Prostanoide
- Phosphodiesterase-5 (PDE5-)Inhibitoren